FITC标记的γ-5羧酸合成酶抗体
产品名称： FITC标记的γ-5羧酸合成酶抗体
英文名称： Anti-P5CS/FITC
产品编号： HZ-
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749 点击查看
- 传真 : 点击查看
- 邮箱 : www.shzbio.net
- 二维码 : 点击查看
Rabbit Anti-P5CS/FITC Conjugated antibody
FITC标记的γ-5羧酸合成酶抗体
| 英文名称 | Anti-P5CS/FITC |
| 中文名称 | FITC标记的γ-5羧酸合成酶抗体 |
| 别 名 | 2810433K04Rik; AI429789; Aldehyde dehydrogenase 18 family member A1; Aldehyde dehydrogenase 18A1; Aldehyde dehydrogenase family 18 member A1; ALDH18A1; Delta 1 pyrroline 5 carboxylate synthetase; Delta1 pyrroline 5 carboxlate synthetase; Gamma-glutamyl kinase; Gamma-glutamyl phosphate reductase; GK; Glutamate-5-semialdehyde dehydrogenase; Glutamyl-gamma-semialdehyde dehydrogenase; GPR; GSAS; MGC117316; MGC32233; P5CS; P5CS_HUMAN; PYCS; Pyrroline 5 carboxylate synthetase (glutamate gamma semialdehyde synthetase). |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Dog, Pig, Cow, Horse, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 87kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human P5CS |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene. [provided by RefSeq, Jul 2008] Function: Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine. Subcellular Location: Mitochondrion inner membrane. DISEASE: Defects in ALDH18A1 are the cause of mental retardation-joint hypermobility-skin laxity with or without metabolic abnormalities (MRJHSL) [MIM:612652]. Clinical manifestations include microcephaly, progressive neurologic dysfunction, mental retardation, progeroid appearance, joint hypermobility, skin laxity and hyperelasticity, cataracts. Some patients manifest metabolic disturbances such as hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia. Similarity: In the N-terminal section; belongs to the glutamate 5-kinase family. In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family. Database links: Entrez Gene: 514759 Cow Entrez Gene: 477781 Dog Entrez Gene: 100061213 Horse Entrez Gene: 5832 Human Entrez Gene: 56454 Mouse Entrez Gene: 100151977 Pig Entrez Gene: 361755 Rat Omim: 138250 Human SwissProt: P54886 Human SwissProt: Q9Z110 Mouse Unigene: 500645 Human Unigene: 233117 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
该基因是醛脱氢酶家族的成员，编码具有γ-谷氨酰激酶和γ-谷氨酰磷酸还原酶活性的双功能ATP和NADPH依赖的线粒体酶。该编码蛋白催化谷氨酸还原为δ1-5-羧酸盐，这是脯氨酸、鸟氨酸和精氨酸新生物合成的关键步骤。该基因的突变导致高氨血症、低鸟氨酸血症、低三核苷酸血症、低精氨酸血症和低脯氨酸血症，并可能与神经变性、白内障和结缔组织疾病有关。另一种剪接转录变体，编码不同的异构体，已被描述为该基因。[ RefSeq，JUL 2008 ]